negin salemi

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Purpose: Due to limited and heterogeneous reports, there is still no information on the relationship between the type of inborn errors of immunity (IEIs) and two main parameters including the incidence rate and severity of COVID-19. Therefore, this study aims to introduce IEIs that may increase the chance of SARS‑CoV‑2 infection or have the highest probability of risk for a severe type of COVID-19 before vaccination. Methods: Data was collected through medical documents obtained from 15 patients with different IEIs and interviews with those who experienced COVID-19 before vaccination and enrolled in our IEIs registry. Results: Only three patients (20%), two men with Bruton Disease (BD) and one woman with Autosomal Recessive Hypogammaglobinemia (ARH), experienced severe-prolonged COVID-19. The frequency of patients with moderate and severe COVID-19 was equal (13.33%). In the female group, one patient with Common Variable Immunodeficiency (CVID) and one patient with Combined Immunodeficiency (CID) had moderate and severe forms of COVID-19, respectively. In contrast, both men who experienced moderate and severe COVID-19 were suffering from BD. Conclusion: Although a small number of patients with BD, CID, and ARH experienced severe and severe- prolonged COVID-19, no formation of cytokine storm was observed, probably owing to IVIG therapy and congenital disorders in the formation or function of cells producing cytokines (B&T cells). Therefore, IEIs not only cannot be a crucial risk factor for COVID-19 but also may provide a great research opportunity to find potential therapies for the prevention of severe COVID-19 through the temporary suppression of some immune system components.